Congenital myotonic dystrophy: ventriculomegaly and shunt considerations for the pediatric neurosurgeon.

PURPOSE: Ventriculomegaly in infants with congenital myotonic dystrophy (CDM) is common, and the neurosurgical determination of shunting is complex. The natural history of CDM-associated ventriculomegaly from prenatal to natal to postnatal stages is poorly known. The relationship between macrocephaly and ventriculomegaly, incidence of shunt necessity, and early mortality outcomes lack pooled data analysis. This study aims to review clinical features and pathophysiology of CDM, with emphasis on ventriculomegaly progression, ventriculomegaly association with macrocephaly, and incidence of shunting. METHODS: This is a literature review with pooled data analysis and case report. RESULTS: One hundred four CDM patients were reviewed in 13 articles that mentioned CDM with ventriculomegaly and/or head circumference. Data was very limited: only 7 patients had data on the presence or absence of prenatal ventriculomegaly, 97 on ventriculomegaly at birth, and 32 on whether or not the ventricles enlarged post-natally. Three patients of 7 (43 %) had pre-natally diagnosed ventriculomegaly, 43 of 97 (44 %) had ventriculomegaly at birth, and only 5 of 32 (16 %) had progressive enlargement of ventricles post-natally. Only 5 of 104 patients had a documented shunt placement: 1 for obstructive, 1 for a post-hemorrhagic communicating, 2 for a communicating hydrocephalus without hemorrhage, and 1 with unknown indication. Of 13 macrocephalic patients with data about ventricular size, 12 had ventriculomegaly. CONCLUSIONS: Ventriculomegaly occurs regularly with CDM but most often does not require CSF diversion. Decisions regarding neurosurgical intervention will necessarily be based on limited information, but shunting should only occur once dynamic data confirms hydrocephalus.

Seizure control after subtotal lesional resection.

Reports on seizure outcomes following surgery for lesional epilepsy consistently cite extent of resection as a significant predictor of outcome. Unfortunately, gross-total resection is not technically feasible in all cases of medically refractory tumor-associated epilepsy. Here, the authors present the case of a 4-year-old girl whose epilepsy was medically controlled after 1-stage electrocorticography-guided subtotal resection (STR) of a large diffuse protoplasmic astrocytoma. They also review the modern literature on epilepsy associated with brain tumors. Outcomes are compared with those following surgical treatment of focal cortical dysplasia and vascular lesions. Gross-total lesional resection shows significant superiority across pathologies and anatomical regions. Despite a considerable number of STRs yielding seizure freedom, other favorable treatment factors have not been defined. Although gross-total lesional resection, if possible, is clearly superior, tailored surgery may still offer patients a significant opportunity for a good outcome. Treatment factors yielding successful seizure control following STR remain to be fully elucidated.

Selective dorsal rhizotomy for spasticity not associated with cerebral palsy: reconsideration of surgical inclusion criteria.

Children with spastic diplegia from cerebral palsy (CP) experience measurable improvement in their spasticity and motor function following selective dorsal rhizotomy (SDR). The role of this operation in the treatment of other spasticity causes is less well defined. A literature review was undertaken to survey outcomes from SDRs performed outside the CP population. Multiple sclerosis was the most common diagnosis found, accounting for 74 of 145 patients described. Selective dorsal rhizotomies have also been reported in patients with traumatic brain and spinal cord injuries, ischemic and hemorrhagic stroke, neurodegenerative disease, hypoxic encephalopathy, and other causes of spasticity. Outcomes from surgery are generally described as favorable, although postoperative assessments and follow-up times are not standardized across reports. Long-term outcomes are sparsely reported. Larger numbers of patients and more detailed outcomes data have the potential to form a basis for expanding the inclusion criteria for SDR.

Complications associated with molding helmet therapy for positional plagiocephaly: a review.

Molding helmet therapy is a widely accepted treatment for positional plagiocephaly that is generally considered to be low risk. Multiple large outcome studies have shown good results, but adverse events are rarely reported. The literature on helmet therapy was reviewed to clarify the clinical experience with associated complications. Although significant complications were extremely rare, there was a large degree of variability in detection of lesser problems such as minor skin irritation. Patients with a primarily brachycephalic morphology may be at higher risk for poorly fitting orthoses. Most reported complications are minor and self-limited. Maintenance of good helmet hygiene appears to be the most effective strategy for reducing or eliminating complications.

Tuberculosis infection presenting as brain abscess in an immunocompromised host.

Despite advances in modern treatment, tuberculosis remains an ever-present problem. With the HIV epidemic, the prevalence of tuberculosis has risen. Contributing to this trend has been the development of multi-resistant tuberculosis strains, as well as rising immigration from nations where tuberculosis is endemic. Although tuberculosis most commonly manifests as a pulmonary process, milary tuberculosis is also on the rise. Neurotuberculosis, a rare but dangerous progression of pulmonary tuberculosis, remains a difficult diagnostic and treatment dilemma to practitioners both in the United States and abroad. We describe an unusual presentation of neurotuberculosis infection in a previously asymptomatic patient. Early recognition and treatment of intracranial tuberculosis is important if mortality is to be prevented.

Intrathecal colistin for treatment of highly resistant Pseudomonas ventriculitis. Case report and review of the literature.

Nosocomial infections with organisms resistant to multiple antibiotic agents represent an evolving challenge in the intensive care setting, particularly in patients requiring surgical diversion of cerebrospinal fluid. The authors present the case of a 51-year-old woman who endured protracted hospitalization and required multiple surgeries including placement of a ventriculoperitoneal shunt. The shunt subsequently became colonized with Pseudomonas aeruginosa, which demonstrated intermediate sensitivity to amikacin and full resistance to all other antibiotics tested 'After failing to respond to intravenous imipenem as well as intravenous and intrathecal amikacin, the patient was successfully treated with intravenous and intrathecal colistin. Colistin is a polymyxin-type antibiotic, rarely used outside of topical application because of reported nephrotoxicity associated with parenteral administration. With activity limited to Gram-negative organisms, colistin is bactericidal by directly disrupting the structure of cell membranes. Authors of a few case reports in the literature have described successful treatment of various ventriculitis with the intrathecal administration of colistin. With bacterial resistances outpacing the pharmaceutical industry's ability to develop novel antibiotics, colistin represents an important alternative in situations involving multidrug-resistant organisms.

Meningiomas of the pediatric skull base: a review.

Pediatric skull base meningiomas are rare and complex clinical entities. Meningioma is a relatively uncommon brain tumor in children, and only ∼ 27% involve the skull base. Some evidence suggests that these tumors are more likely to be atypical or malignant in children than adults. The absence of female preponderance in pediatric meningiomas is reflected in the skull base subpopulation. Skull base meningiomas in children are most likely to be found in the anterior or middle fossa base, or involving the orbit and optic nerve sheath. Petroclival, suprasellar/parasellar, cerebellopontine angle, cavernous sinus, and foramen magnum tumors are very rare. Meningiomas constitute a small proportion of reported cases of pediatric skull base pathology, and they are entirely absent from many case series. Initial gross total resection is consistently associated with superior outcomes. Surgical approaches to the pediatric skull base must take additional factors into consideration including relatively smaller anatomy, immature dentition, incompletely aerated sinuses and air cells, and altered configurations of structures such as the pterional bony complex. Multidisciplinary expertise is essential to optimizing treatment outcomes.

Endoscopic Endonasal Repair of Congenital Defects of the Anterior Skull Base: Developmental Considerations and Surgical Outcomes.

Techniques of endoscopic endonasal surgery, initially developed primarily for intracranial neoplasms, have been adapted to treat a wide variety of pathologies previously addressed with open craniotomy including congenital and acquired defects of the anterior skull base. Congenital defects can lead to herniation of leptomeninges containing cerebrospinal fluid alone or with brain tissue. Specific types of encephalocele can be defined on the basis of the associated abnormal bony anatomy. Endoscopic endonasal surgery represents a relatively recent development in the treatment of these entities. Technical considerations include relatively younger age range of the patient population, dimensions of preexisting bony defect, volume of herniated meninges and brain tissue, and distorted anatomy from abnormal development of the affected craniofacial skeleton. Recent highly detailed anatomical studies have quantitatively verified the utility of endoscopic endonasal surgery in the pediatric population. Particular attention has been directed toward adequacy of nasoseptal flap reconstruction in pediatric patients. Several reports have described patients with encephalocele of the anterior cranial fossa successfully treated with endoscopic surgery. The literature on endoscopic repair of congenital encephalocele is reviewed. Outcomes have generally been reported as favorable, although long-term follow-up and systematic studies have not been pursued.

Zenker diverticulum: a rare complication after anterior cervical fusion.

Esophageal injury is a rare but serious complication occurring after anterior cervical spine surgery. Pharyngoesophageal, or Zenker, diverticulum is an acquired outpouching of the pharyngeal musculature just proximal to a functional esophageal stricture, clinically manifesting as dysphagia, aspiration, and weight loss. We report a case in which a patient developed a pharyngoesophageal diverticulum, accompanied by retropharyngeal abscess, first identified 2 years after a 3-level anterior cervical fusion with allograft and anterior plating. The inferior portion of the cervical plate was dislodged anteriorly. In addition, the patient harbored pulmonary and mediastinal infection at the time of presentation. Despite incision and drainage of the abscess with repair of the diverticulum, the patient died in the early postoperative period. Prompt recognition and frequent follow-up, as well as patient education, may prevent this catastrophic complication.